scholarly journals Alternating and Concurrent True Hyperkalemia and Pseudohyperkalemia in Adult Sickle Cell Disease

2021 ◽  
Vol 12 (2) ◽  
pp. e0018
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Tubular Dysfunction ◽  
Renal Tubular Dysfunction ◽  
Cell Disease ◽  
Hemolytic Crisis ◽  
Renal Tubular ◽  
Potassium Binders ◽  
Sodium Zirconium Cyclosilicate

Sickle cell disease (SCD) predisposes the patient to recurrent episodes of acute painful hemolytic crisis. Sickle cell nephropathy (SCN) is not uncommon in adult patients, and renal manifestations of SCN include renal ischemia, microinfarcts, renal papillary necrosis, and renal tubular abnormalities with variable clinical presentations. Intravascular hemolysis and reduced glomerular filtration rate with renal tubular dysfunction predispose to true hyperkalemia. Hemolytic crisis can be complicated by sepsis, leading to significant degrees of thrombocytosis, and thrombocytosis is a well-defined cause of pseudohyperkalemia. We describe a 40-year-old African American male patient with sickle cell anemia who exhibited alternating episodes of true hyperkalemia and pseudohyperkalemia, during consecutive hospital admissions. Clearly, true hyperkalemia is a potentially lethal condition. At the same time, the institution of inappropriate and intensive treatment of pseudohyperkalemia leading to severe hypokalemia is also potentially lethal. The need for this caution is most imperative with the recent introduction of the safer and more potent potassium binders, patiromer and sodium zirconium cyclosilicate.

scholarly journals MO218ALTERNATING EPISODES OF TRUE HYPERKALEMIA AND PSEUDOHYPERKALEMIA IN ADULT SICKLE CELL DISEASE - A NEPHROLOGIST'S DILEMA

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Macaulay Onuigbo ◽  
Sarah Sherman ◽  
Heng Tan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Serum Potassium ◽  
Hospital Admissions ◽  
Adult Patients ◽  
Electrolyte Imbalance ◽  
Cell Disease ◽  
Clinical Scenarios ◽  
Hemolytic Crisis ◽  
Potassium Binders

Abstract Background and Aims To illustrate the phenomenon of alternating true hyperkalemia and pseudohyperkalemia in adult sickle cell disease. Method Case Report Results Sickle cell disease (SCD) predisposes the patient to recurrent episodes of acute painful hemolytic crisis. Sickle cell nephropathy (SCN) is not uncommon in adult patients. The presence of sickled erythrocytes in the renal medullary vessels is the hallmark of the disease and renal manifestations include renal ischemia, microinfarcts, renal papillary necrosis and renal tubular abnormalities with variable clinical presentations. Furthermore, acute hemolytic crisis can be complicated by sepsis. Hemolysis, specifically, intravascular hemolysis, can produce hyperkalemia. Additionally, reduced glomerular filtration rate from SCN predisposes to hyperkalemia. Pseudo-hyperkalemia was first reported by Hartmann and Mellinkoff in 1955 as a marked elevation of serum potassium levels in the absence of clinical evidence of electrolyte imbalance. In pseudohyperkalmia, simultaneously estimated serum potassium exceeds plasma potassium by >0.4 mmol/L. This is often associated with moderate to severe thrombocytosis or leukocytosis. Clearly, hyperkalemia is a potentially lethal condition. At the same time, the institution of inappropriate treatment of pseudo-hyperkalemia leading to hypokalemia is also equally potentially lethal. We describe a 40-yo African American male patient with sickle cell anemia who exhibited alternating episodes of hyperkalemia and pseudo-hyperkalemia, during consecutive hospital admissions. Pseudohyperkalemia was associated with severe thrombocytosis complicating sepsis. EKG was normal despite measured serum potassium of 6.7 mmol/L (Figure). Conclusion We believe that this is the first report of adult SCD demonstrating alternating cycles of true hyperkalemia and pseudo-hyperkalemia at different times. We must draw attention to the new availability of the new potassium binders, Patiromer and sodium zirconium cyclosilicate. We would advocate for caution in the use of these potent potassium binders and to always give consideration to the presence of pseudo-hyperkalemia under appropriate clinical scenarios. We posit that providers managing adult patients with sickle cell disease must be aware of such a phenomenon to avoid the dangers of overtreatment of episodes of pseudo-hyperkalemia in such patients.

scholarly journals Renal Tubular Dysfunction in Sickle Cell Disease

2013 ◽  
Vol 38 (1) ◽  
pp. 1-10 ◽  
Author(s):  
Geraldo B. Silva Junior ◽  
Ana Patrícia F. Vieira ◽  
Amanda X. Couto Bem ◽  
Marília P. Alves ◽  
Gdayllon C. Meneses ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Tubular Dysfunction ◽  
Renal Tubular Dysfunction ◽  
Cell Disease ◽  
Renal Tubular

scholarly journals Hemoglobin inhibits albumin uptake by proximal tubule cells: implications for sickle cell disease

2017 ◽  
Vol 312 (6) ◽  
pp. C733-C740 ◽  
Author(s):  
Megan L. Eshbach ◽  
Amandeep Kaur ◽  
Youssef Rbaibi ◽  
Jesús Tejero ◽  
Ora A. Weisz
Keyword(s):  
Sickle Cell Disease ◽  
Proximal Tubule ◽  
Sickle Cell ◽  
Prolonged Exposure ◽  
Tubular Dysfunction ◽  
Cell Disease ◽  
Tubular Proteinuria ◽  
Proximal Tubule Cells ◽  
Dimer Interface ◽  
Hemolytic Crisis

Proximal tubule (PT) dysfunction, including tubular proteinuria, is a significant complication in young sickle cell disease (SCD) that can eventually lead to chronic kidney disease. Hemoglobin (Hb) dimers released from red blood cells upon hemolysis are filtered into the kidney and internalized by megalin/cubilin receptors into PT cells. The PT is especially sensitive to heme toxicity, and tubular dysfunction in SCD is thought to result from prolonged exposure to filtered Hb. Here we show that concentrations of Hb predicted to enter the tubule lumen during hemolytic crisis competitively inhibit the uptake of another megalin/cubilin ligand (albumin) by PT cells. These effects were independent of heme reduction state. The Glu7Val mutant of Hb that causes SCD was equally effective at inhibiting albumin uptake compared with wild-type Hb. Addition of the Hb scavenger haptoglobin (Hpt) restored albumin uptake in the presence of Hb, suggesting that Hpt binding to the Hb αβ dimer-dimer interface interferes with Hb binding to megalin/cubilin. BLAST searches and structural modeling analyses revealed regions of similarity between Hb and albumin that map to this region and may represent sites of Hb interaction with megalin/cubilin. Our studies suggest that impaired endocytosis of megalin/cubilin ligands, rather than heme toxicity, may be the cause of tubular proteinuria in SCD patients. Additionally, loss of these filtered proteins into the urine may contribute to the extra-renal pathogenesis of SCD.

scholarly journals Risk-Score Based Strategy to Minimize Antibiotic Exposure in Children With Sickle Cell Disease and Fever

2021 ◽  
Author(s):  
Elena Maria Rincón-López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández-Sampelayo Matos ◽  
David Aguilera-Alonso ◽  
Eva Dueñas Moreno ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Risk Score ◽  
Bacterial Infections ◽  
Hospital Admissions ◽  
Empirical Therapy ◽  
Antibiotic Exposure ◽  
Cell Disease ◽  
Optimal Score ◽  
A Prospective Study

Abstract Severe bacterial infections (SBI) have become less frequent in children with sickle cell disease (SCD) in the last decades. However, because of their potential risk of SBI, they usually receive empirical therapy with broad-spectrum antibiotics when they develop fever and are hospitalized in many cases. We performed a prospective study including 79 SCD patients with fever [median age 4.1 (1.7–7.5) years, 78.5% males; 17 of the episodes were diagnosed with SBI and 4 of them were confirmed] and developed a risk score for the prediction of SBI. The optimal score included CRP > 3 mg/dl, IL-6 > 125 pg/ml and hypoxemia, with an AUC of 0.91 (0.83–0.96) for the prediction of confirmed SBI and 0.86 (0.77–0.93) for possible SBI. We classified the patients in 3 groups: low, intermediate and high risk of SBI. Our risk-score based management proposal could help to safely minimize antibiotic treatments and hospital admissions in children with SCD at low risk of SBI.

scholarly journals Air Drep—A Retrospective Study Evaluating the Influence of Weather Conditions and Viral Epidemics on Vaso-Occlusive Crises in Patients with Sickle Cell Disease Living in French Guiana

2019 ◽  
Vol 16 (15) ◽  
pp. 2724
Author(s):  
Marie-Claire Parriault ◽  
Claire Cropet ◽  
Aniza Fahrasmane ◽  
Stéphanie Rogier ◽  
Michaël Parisot ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
French Guiana ◽  
Hospital Admissions ◽  
Weather Conditions ◽  
Therapeutic Education ◽  
Targeted Prevention ◽  
Prevention Measures ◽  
Cell Disease ◽  
Emergency Visits

(1) Objectives: French Guiana is the French territory most affected by sickle cell disease (SCD). This study investigates the associations between different environmental factors relative to climate, infectious outbreaks, and emergency visits or weekly hospital admissions for vaso-occlusive crisis (VOC). The identification of risk factors would lead to better patient care and patient management, and more targeted prevention and therapeutic education for patients with SCD in French Guiana. (2) Methods: This study was performed using data collected from the medicalized information system and emergency medical records of Cayenne General Hospital, between 1 January 2010 and 31 December 2016. ARIMA models were used to investigate the potential impact of weather conditions and flu epidemics on VOC occurrence. (3) Results: During the study period, 1739 emergency visits were recorded among 384 patients, of which 856 (49.2%) resulted in hospitalization, 811 (46.6%) resulted in hospital discharge, and 72 (4.2%) in another orientation. Decreased temperature and decreased humidity were both independent factors associated with an increase of VOC cases (p = 0.0128 and p = 0.0004, respectively). When studying severe VOC (leading to hospitalization, with or without prior emergency visit), 2104 hospital admissions were recorded for 326 patients. The only factor associated with severe VOC, in the multivariate analysis, was flu epidemics (p = 0.0148). (4) Conclusions: This study shows a link between climate, flu epidemics, and VOC in French Guiana. Patient’s awareness of risks related to climate and flu epidemics should be encouraged, as home prevention measures can help avoid painful crises. Moreover, physicians should encourage patients to get immunized for influenza every year.

Incomplete renal tubular acidosis in sickle cell disease

1972 ◽  
Vol 41 ◽  
pp. 149-156 ◽  
Author(s):  
J.P. Goossens ◽  
L.W.Statius Van Eps ◽  
H. Schouten ◽  
A.L. Giterson
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Renal Tubular Acidosis ◽  
Cell Disease ◽  
Renal Tubular

Changing Pattern of Hospital Admissions of Children With Sickle Cell Disease Over the Last 50 Years

2011 ◽  
Vol 33 (7) ◽  
pp. 491-495 ◽  
Author(s):  
Thomas G. Day ◽  
Swee Lay Thein ◽  
Emma Drasar ◽  
Moira C. Dick ◽  
Susan E. Height ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Cell Disease

Renal Involvement in Saudi Patients with Sickle Cell Disease. A Single Institution Experience.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3734-3734
Author(s):  
Hikmat N. Abdel-Razeq ◽  
Ali Bajouda ◽  
Manar Khalil ◽  
Muneera Al-Shareef ◽  
Nauman Siddiqi
Keyword(s):  
Sickle Cell Disease ◽  
Serum Creatinine ◽  
Sickle Cell ◽  
Urine Analysis ◽  
Renal Involvement ◽  
Urine Volume ◽  
Cell Disease ◽  
Study Results ◽  
Renal Tubular ◽  
Saudi Patients

Abstract Background: Renal involvement in patients with sickle cell disease has been reported to be as high as 20%. This can take the form of proteinuria, decreased GFR, isothenuria or hematuria. Our own clinical observation of patients attending our sickle cell clinic or admitted to the medical ward at our institution suggests lower rates of renal involvement than previously reported. In this report, we evaluate Saudi patients with sickle cell disease (SS) at our institution for renal involvement. Patients and Methods: Consecutive afebrile patients attending sickle cell clinic or admitted to inpatient wards were screened. Serum creatinine, urine analysis and 24 hr urine collection for creatinine clearance (GFR) and proteinuria were performed. Data on demographics and co-morbidities (DM, HTN, hepatitis B and C) and medications were obtained. Analysis was performed using SPSS 10.0®. Results: 62 patients were available for study; results are summarized in tables. Seven of 48 patients (15%) had proteinuria >300mg/d while two (4.2%) had proteinuria of more than 1.0 gram/24 hrs. Nearly 20% had GFR < 60ml/min. None of the patients had renal tubular acidosis. Serum creatinine was normal in all patients including those with low GFR and proteinuria. Conclusions: Contrary to our initial believes, significant percentage of Saudi sickle cell patients, like others, have renal involvement with low GFR and proteinuria. Serum Creatinine alone doesn’t indicate this decline in renal function. We recommend annual evaluation for proteinuria and GFR determination in all sickle cell patients, and those at risk should be aggressively managed with nephroprotective agents like angiotensin converting enzyme (ACE) inhibitors. Age (yrs) M:F BMI Hepatitis C Hydroxyurea NSAID 22.1+/−8.1 23:39 19.3+/−5.1 9 (17.3%) 29 (58%) 33 (66%) Urine Volume (ml/24 hrs) Specific Gravity Serum Creatinine (u mol/L) GFR Proteinuria (mg/24hrs) 1927 +/− 780 1.011 +/− 0.005 40.6 +/− 12.9 113.7 +/− 47.7 256 +/− 371 (20–2390)

scholarly journals Associations between environmental factors and hospital admissions for sickle cell disease

Haematologica ◽  
2016 ◽  
Vol 102 (4) ◽  
pp. 666-675 ◽  
Author(s):  
Frédéric B. Piel ◽  
Sanjay Tewari ◽  
Valentine Brousse ◽  
Antonis Analitis ◽  
Anna Font ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Environmental Factors ◽  
Sickle Cell ◽  
Hospital Admissions ◽  
Cell Disease

Family caregiving for adults with sickle cell disease and extremely high hospital use

2016 ◽  
Vol 21 (12) ◽  
pp. 2893-2902 ◽  
Author(s):  
Shan-Estelle Brown ◽  
Daniel F Weisberg ◽  
William H Sledge
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Family Caregiving ◽  
Hospital Admissions ◽  
Early Death ◽  
Romantic Partners ◽  
Cell Disease ◽  
Parental Obligations ◽  
Resilience Training ◽  
Hospital Use

This study investigated coping with chronic illness in the adult patient–caregiver relationship for sickle cell disease, marked by debilitating acute and chronic pain. One-on-one interviews ( N = 16) were conducted with eight primary caregivers of eight adults with extremely high hospital use, severe sickle cell disease with hospital admissions several times monthly over successive years. Caregivers were predominantly parents; two were romantic partners. Caregivers attributed disruptions to the disease’s variability, tensions in how much support to give, and adults’ inability to fulfill parental obligations. Both groups expressed fears of patients’ increasing age, declining health, and early death. Targeted counseling and resilience training is recommended.

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